Combined hepatocholangiocarcinoma: case-series and review of literature.

Background and aim: Combined hepatocholangiocarcinoma (CHCC) is an infrequent primary hepatic malignancy with no clearly defined diagnostic criteria, poorly studied natural history, and no guidelines regarding therapy. In this study we attempted to address this need and review our experience.

Methods and results: We performed a retrospective review of all CHCC cases at our institute over the last 10 yr. Eight cases were identified; histological and immunohistochemical criteria used for diagnosis were defined. Patients characteristics were: median age 65 yr (range 47-80); five females; risk factors-cholelithiasis (n = 4) and cirrhosis due to chronic viral hepatitis B and C (n = 1). Abdominal pain (n = 6), hepatomegaly (n = 4), and elevated CA 19-9 >40 U/mL (n = 4/5) were frequent. Early TNM stage (I and II) compared with advanced disease (III and IV) correlated with higher overall survival on univariate analyses [37 and 6 mo respectively (p = 0.011)]. Median overall survival was significantly higher in patients who underwent potentially curative resection (23 mo, range 4-48+) compared with patients who underwent non-surgical therapies such as transcatheter arterial chemoembolization and chemotherapy (2 mo, range 1-8) (p = 0.0357, one-sided exact log-rank test).

Conclusions: Chronic inflammation and cirrhosis may play a role in pathogenesis of CHCC. Surgical resection and early stage at diagnosis predict longer survival.